肺癌を伴う亜急性小脳変性症の1剖検例—特に小脳病変の分布について

AN AUTOPSY CASE OF CARCINOMATOUS SUBACUTE CEREBELLAR DEGENERATION:ON DISTRIBUTION OF CEREBELLAR CORTICAL LESIONS

　抄録　悪性腫瘍と小脳変性症が合併した症例は欧米ではかなりの報告例が見られるが，本邦ではわずか数例の剖検例が報告されているにすぎず，しかも神経病理的に病変の分布を詳細に検討した報告はほとんどない。我々は肺癌を伴う亜急性小脳変性症の1例の小脳病変に関する詳細な形態計測をおこない，1自験例を含め，アルコール性およびdiphenylhydantoin中毒性小脳変性症と文献的に比較した。Purkinje細胞の脱落は広汎であったが，病変の程度には特徴的な局在があることを指摘した。すなわち，Purkinje細胞の脱落の最も強い部位は，虫部では中心小葉，山頂で，小脳半球では中心小葉翼と四角小葉であり，一般的に言って小脳の上面で前部に病変が強い傾向が明らかにうかがえる。小舌が比較的病変を免れているのは注目に値する。髄体に近い部分の活樹では細胞がよく残存し，小脳表面に近い部の活樹ではPurkinje細胞はより高度な脱落を示す傾向がみられる。diphenylhydantoin中毒やアルコール中毒症とは病変分布の点で異なり，このように詳細な形態計測により，種々の小脳皮質変性症この病変の異同を比較検討することは，個々の小脳変性症の病因解明への糸口を見出すきっかけとなると思われる。

A 46-year-old man developed sudden dysarthria and atactic gait and was noted to be unable to get up even on the bed about one year prior to his death. By following several days, he started tohave scanning speech, nausea, trancal ataxia and dysmetria in succession. The cerebro-spinal fluid yielded moderate pleocytosis. There were no sen-sory disturbance, pathological reflexes and Rom-berg's sign. Half a year later, submandibular tumor was noted. The biopsy showed metastatic small cell undifferentiated carcinoma, presumably of pulmonary origin, and paraneoplastic cerebellar degeneration was suspected. He died of broncho-pneumonia, superimposed on lung cancer on February 25 in 1979. The necropsy showed a large tumor in the right lung which was histologically verified small cell undifferentiated carcinoma (so-called oat-cell carcinoma). The cerebellum disclosed diffuse cortical atrophy, chiefly of Purkinje cell type. Moderate demyelination with reparative gliosis and foamy macrophages was seen in the white matter, which was considered secondary to cortical devastation. The morphometric study on Purkinje cell loss showed interesting distribution of the lesions. The severely affected portions were the central lobe and culmen in the vermis, and the ala lobuli centralis and quadrangular lobe in the hemisphere, respectively. The lingula was strikingly spared. The finding was compared with that of other cerebellar disease in reviewing the literature.